Synovial sarcoma (SS) is a rare type of cancer characterized by a variable degree of epithelial differentiation and formation of the SS18-SSX fusion gene. The SS18-SSX fusion gene results from the chromosomal translocation t (X; 18) and most commonly arises within the deep soft tissue of extremities. Here, we describe a case report of an adult presenting with spindle cell-type primary pulmonary SS (PPSS) of the right thorax region, and review the clinicopathological features of PPSS according to the literature. (Am J Transl Med 2020. 4:197-204).